Biliary Atresia Clinical Care Program

Treatment

Surgery: Kasai procedure

The Kasai procedure is the preferred treatment for biliary atresia at The Children's Hospital of Philadelphia and has been performed here for more than 30 years. The operation connects the bile draining from the liver directly to the intestinal tract. It is most successful when done early in the disease process.

The specific age to obtain the best results from the Kasai procedure is unknown because biliary atresia is a rare disease and manifests differently in every child. Researchers have reported varying results over the past three decades. Some suggest the Kasai procedure is best completed before the infant is four weeks old, while others say the best rest results are produced when the operation is completed by the time the baby is 8-10 weeks old.

While the Kasai procedure is not a cure for biliary atresia, it is the preferred treatment because it allows a child to grow and remain in good health for several years. A successful Kasai procedure delays the need for a liver transplant. In rare cases, the Kasai can actually eliminate the need for a liver transplant.

Nutrition

With biliary atresia, not enough bile reaches the intestine to assist with the digestion of fats in the diet. Protein deficiencies may occur due to liver damage. Vitamin deficiencies may also occur. Children with liver disease require more calories than a normal child because they do not absorb the same amount of calories and nutrition. Your physician may recommend that a pediatric nutritionist make recommendations regarding your child's diet. Nutritional guidelines may include the following:

Liver transplant

A liver transplant removes the damaged liver and replaces it with a new liver from a donor. The new liver can be:

After surgery, the new liver begins functioning and the child’s health often improves quickly.

After a liver transplant, children will need to take medications to prevent the body from rejecting the new organ. Rejection occurs due to one of the body's normal protective mechanisms that helps fight against an invasion of viruses, tumors or other foreign substances. Anti-rejection medications are taken in order to prevent this normal response of the body from fighting against the transplanted organ.

Frequent contact with the physicians and other members of the transplant team is crucial after a liver transplant.

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